Clinical and anatomic analysis of children with congenital bridging bronchus malformation / 中华实用儿科临床杂志

ABSTRACT

Objective To analyze the clinical and anatomic characteristics of bridging bronchus (BB) malformation in children,and to explore its diagnostic strategy,treatment and risk factors.Methods A retrospective study was performed in 23 pediatric patients with BB who were admitted to Guangzhou Women and Children's Medical Center form May 2010 to October 2016.All their clinical features,imaging examination,treatment and prognosis were reviewed and analyzed.Results (1) Among the 23 patients with BB,15 were males and 8 were females.The age range of onset was 0 to 4 years old with a median age of 3.17 months.(2)The main clinical manifestations were cough (23/23 cases,100.0％),recurrent wheezing (20/23 cases,87.0％),cyanosis (8/23 cases,34.8％) and feeding diffficulty/slow body weight growth (6/23 cases,24.6％).(3) There were 17 cases of type Ⅰ and 4 cases of type Ⅱ according to Wells,and the other 2 cases were anterior BB.There were 2 cases with atypical anatomical morphology in type Ⅰ and type Ⅱ respectively.(4) Twenty-one cases were associated with bronchial stenosis in which the origin of left main bronchus complicated with BB stenosis was the most common(5 cases).Bronchoscopy revealed bronchomalacia in 5 of the 16 patients.Sixteen patients associated with cardiac or vascular anomalies,11 of whom were compound anomalies,and the most common type was sling left pulmonary artery (SLPA) (12 cases).(5) Nine of 12 patients with SLPA received surgical management,ages ranging from 1 month to 8 years old.The respiratory symptoms remitted gradually or disappeared from 4 to 17 months after surgical management.Two died and one has been lost to follow-up among the remaining 3 patients with SLPA.Conclusions BB is a rare tracheobronchial malformation that is often associated with stenosis or bronchomalacia,and cardiac or vascular anomalies.The severity of the disease mainly depends on whether there is airway stenosis or malacia and its extent.The definitive diagnosis of BB requires the combination of different imaging modalities.The combination with simple SLPA could get better curative effect.